Double lung transplantation for diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.

Overview

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare cause of obstructive lung disease and a proposed precursor of pulmonary carcinoid tumors. With increased awareness of this disease, an accumulating number of cases have been reported. Patients may have progressive shortness of breath due to small airway obstruction. There is limited information on treatment of the disease, particularly for those with progressive disease. Here we report 2 patients who were treated at our institution with double lung transplantation. Both patients were female nonsmokers with a clinical presentation of cough and dyspnea. Multiple bilateral lung nodules were identified on high-resolution computed tomography and pulmonary function tests showed obstructive pattern in both patients. The diagnoses of DIPNECH were made by histologic evaluation of the explanted native lungs. These 2 patients are in stable condition following double lung transplantation at the time of this report. DIPNECH is increasingly recognized due to improved sensitivity of investigative imaging and increased awareness of the entity. Considering the malignant potential and lack of effective treatment of the disease, lung transplantation could be potentially an effective treatment option for these patients.